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ACP Hematology > L12 Multiple myeloma (plasma cell myeloma) and lymphoproliferative neoplasms > Flashcards

L12 Multiple myeloma (plasma cell myeloma) and lymphoproliferative neoplasms Flashcards

1
Q

Clonal plasma cell proliferation in BM/ extra-medullary region secreting protein =

A

plasma cell myeloma

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2
Q

What is the most common form of plasma cell myeloma?

A
  1. intact Ig (light chain + heavy chain)

2. free light chain only: can be secreted in urine: Bence Jones protein

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3
Q

What are the clinical characteristics of plasma cell myeloma? (3)

A
  1. lytic bone lesions
  2. plasma cell accumulation in bone marrow
  3. presence of monoclonal protein in the serum and urine
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4
Q

Clincal presentations of plasma cell myeloma? (in blood) (3)

A
  1. incidental finding of reversed A:G ratio (albumin: globulin ration should > 1 normally)
  2. Elevated ESR (erythrocyte sedimentation rate) due to increased Ig in blood
  3. hypercalcemia
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5
Q

Clinical presentations of paraproteinemia in plasma cell myeloma?

A
  1. amyloidosis

2. hyperviscosity syndrome (due to increased Ig): headache, stroke

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6
Q

Clincal presentations of end orgain damage in plasma cell myeloma?

A

CRAB

  1. Calcium too high, due to release of osteoclast activating factors by tumor cells
  2. Renal insufficiency: Cr>177
  3. Anemia: Hb<10
  4. Bone lesion: Osteolytic lesion/ spinal cord compression
  5. Others: recurrent bacterial infections ..
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7
Q

What are the 3 categories of plasma cell dyscrasia?

and sub-types

A
  1. MGUS
    - monoclonal gammopathy of undetermined significance
  2. Plasma cell myeloma - symptomatic and asymptomatic
  3. Plasmacytoma - bone and extra-osseous
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8
Q

Which plasma cell dyscrasia?
Serum M protein (Ig) <30g/L
BM clonal plasma cells < 10%
No ROTI

A

MGUS

monoclonal gammapathy of undetermined significance

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9
Q

Which plasma cell dyscrasia?
Serum M protein (Ig) > 30g/L OR
BM clonal plasma cells > 10%
No ROTI

A

Plasma cell myeloma (=Multiple myeloma) - Asymptomatic

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10
Q 
In symptomatic plasma cell myeloma,
A. M-protein in serum or urine is required for diagnosis
B. BM clonal plasma cells >10% 
C. CRAB clinical presentations appear 
D. Amyloidosis present
A

All except A

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11
Q

Plasmacytoma is a kind of plasma cell dyscrasia, which may progress to?

A

Multiple myeloma (plasma cell myeloma)

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12
Q

What are the 2 main types of plasmacytoma? Briefly describe them.

A
  1. Solitary skeletal
    - single, localised bone tumor with clonal plasma cells - no other lesions found in skeletal survery or MRI
    - BM study and clinical features not consistent with plasma cell myeloma (multiple myeloma)
  2. Extraosseous
    - localized neoplasm in neo-osseous tissues
    - common sites: URT, GIT, LN
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13
Q

What is used to diagnose any plasma cell dyscrasia by looking at the M protein?

A

SPE - serum protein electrophoresis
SIFE - serum immunofixation electrophoresis

SPE: quantify M protein, but don’t specify which M protein
SIFE: characterise M protein (IgD lamda - abnormal)

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14
Q

Most common in SIFE? (sequence)

A

IgG > IgA > FLC (light chain disease)

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15
Q

What method is used to check the light chains in plasma cell dyscrasia?

A
  • urine for Bence Jones proteins
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16
Q

In BM study, what features can be seen in plasma cell dyscrasia?
comment on morphology and immunophenotype.

A
  1. Morphology
    - plasmacytosis > 30% (but variable due to patchy distribution)
    - plasma cell atypia (large, nucleolated, irregular nuclei/multinucleated, anaplasitc)
  2. immunophenotype
    - CD138+
    - CD20-
    - light chain restriction+
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17
Q

What are the markers for prognosis in plasma cell dyscrasia?
state the prognosis of each marker.

A
  1. beta2- microglobulin
  2. albumin
  • high serum beta2- microglobulin = worst prognosis
  • high serum albumin = poor prognosis
18
Q

What are indicators of favorable and unfavorable pronosis in cytogenetics (G-banding and FISH)?

A

Favorable: hyperdiploidy (Ch5,9,15 usually)
Unfavorable: 13q deletion

19
Q

When is treatment required in plasma cell myeloma?

How?

A

When end organ damaged is present;

  • Fit patient: Self stem cells > chemotherapy to destroy BM > cell transplantation
  • Unfit patient: palliative chemotherapy
20
Q

What is lymphoproliferative neoplasm?

A

Clonal proliferation of mature lymphoid cells, characterized by lymphocytosis

21
Q

Compare and contrast CLPD (chronic lymphoproliferative disorders) and lymphoma.

A

Similarity:
- clonal proliferation of mature lymphoid cells

Differences:

  1. Origin
    - CLPD: BM and PB
    - lymphoma: nodals and extranodal tissues
  2. May involve
    - CLPD: LN, spleen, liver
    - lymphoma: CM and PB (leukemic phase)
22
Q

State possible etiology of lymphoproliferative neoplasm?

A
  1. Virus: HTLV-1 (ATLL (Adult T-cell Leukemia/lymphoma)) , EBV (aggressive NK cell lymphoma)
  2. Infection: H.pylori
23
Q

Natural killer cells VS T cells nature?

A

NK cells are cytotoxic CD8+ cells without T cell receptor

24
Q

What are the main immunophenotype of

  1. B cells?
  2. T helper cells?
  3. cytotoxic T cells?
  4. Natural killer cells?
A
  1. B cells: CD20+
  2. T helper cells: CD4+
  3. cytotoxic T cells: CD8+
  4. Natural killer cells: CD56+
25
Q 
Which of the following clinical features of LPD are correct?
A. Male has higher chance to have LPD
B. Mostly before age of 40
C. It can be asymptomatic 
D. Fever and weight loss is possble
E. Lymphadenopathy is possble
F. Small spleen
G. Bone marrow failure, cytopenia
H. Tissue infilatation
A

All except B and F
B: rare before age of 40
F: splenomegaly

26
Q

Name the most common form of lymphoproliferative disease?

What are its dianostic criteria?

A

Chronic lymphocytic leukemia (CLL)

  • > 5x10^9 /L monoclonal lymphocytes in peripheral blood for 3 months
  • > 30% marrow involvement
27
Q 
Chronic lymphocytic leukemia:
A. Small lymphocytes in BM
B. clumped chromatin in BM
C. scanty cytoplasm in CM
D. can transform to large cell lymphoma 
E. CD9 is the immunophenotype invovled in abnormal B cell Ag
A

All excepy E

D: Richter syndrome
E: AbnormalB cell Ag: CD5+, CD23+ ;light chain restriction

  • Normal B cell Ag: CD 19,20,22
28
Q

What kind of cell morphology can be seen in CLL? (Chronic lympcytic leukemia)

A
  • Smudge cells (fragile lymphocytes)
29
Q

There are different prognosis if CLL is due to different genetic mutations.
List examples for each prognosis.

A

Good prognosis: Del13q14.3 (一生一世生）
Intermediate: trisomy 12
Poor prognosis: Del17p13 (實柒一生）, Del11q22

30
Q

What are the possible treatment for CLL?

A

Asymptomatic: watchful waiting
Symptomatic: chemotherapy + anti CD20+ (Rituximab)

31
Q

What are the clinical features (3) of hair cell leukemia?

A
  1. Massive splenomegaly
  2. Monocytopenia
  3. Pancytopenia
32
Q

What are the distinct cell morphology for hair cell leukemia? (2)

A
  • Fried egg lymphocyte

- marrow fibrosis (Trephine)

33
Q

What are the abnormal B cell Ag in hair cell leukemia?

Cytochemistry finding?

A
  • CD11c, CD25, CD103, CD123

* cytochemistry: TRAP+ (Tartrate resistant acid phosphatase, strongly positive)

34
Q

What is the mutataed gene in hair cell leukemia?

A

BRAF V600E (missense mutation)

35
Q

What are the 2 types of Mature T cell neoplasm?

A
  1. T-cell prolymphocytic leukemia (T-PLL)

2. Adult T-cell leukemia/lymphoma (ATLL)

36
Q

What is T-cell prolymphocytic leukemia?

A
  • Proliferation of a mature post-thymic T-cell phenotype

- most common T-cell CLPD

37
Q

Which T cell neoplasm has cytoplasmic blebs as one of the most distinct morphology?

A

T-cell prolymphocytic leukemia

38
Q 
Which of the following about T-PLL are true?
A. 60% of cases CD4+CD8-
B. it is a post-thymic derivation
C. it lack the expression of immature markers such as CD34 and Tdt
D. It presents CD3 and CD7 
E. Lymphadenopathy 
F. Serous effusions
G. Splenomegaly
F. Lymphocytosis
A

All of the above

39
Q

Which virus ATLL is associated with?

A

HTLV-1 (human T-cell lymphotrophic virus type 1)

  • endemic in southwestern Japan, Caribbean basin and central africa
40
Q

Skin lesions
Hypercalcemia
Lytic bone lesions

Which leukemia?

A

ATLL

Adult T cell leukemia/lymphoma

41
Q

What are the most characteristic cell morphologies in ATLL? (2)

A
  1. Convoluted/polylobated nuclear contours - clover leaf nuclei
  2. Nuclear pleomorphism
    3
42
Q

Which of the following about ATLL is incorrect?
A. They are related to CD4/CD8 aberrancies
B. They are presented with CD7
C. They are presented with CD25
D. TCR gene rearrangement is present

A

B
CD7-

CD25++

ACP Hematology (16 decks)

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